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Vulval cancer

Author: Vanessa Ngan, Staff Writer, 2003.

What is vulval cancer?

Vulval (or vulvar) cancer or cancer of the vulva can occur on any part of the female external reproductive system but most often affects the inner edges of the labia majora or the labia minora. Over 90% of vulval cancers are squamous cell carcinomas (SCC). The next most common type of vulval cancer is melanoma but this accounts for less than 5% of all vulval cancers. Other rare vulval cancers are basal cell carcinoma (BCC), Bartholin's gland cancer and extramammary Paget disease.

Who gets vulval cancer and why?

Vulval cancer may affect women of all ages but appears to occur most frequently in women aged between 65–75 years. Human papillomavirus (HPV) appears to be responsible for the development of about 60% squamous cell carcinomas of the vulva.

Some vulval cancers may be preceded by precancerous changes that may last for several years. This precancerous condition of the vulva is known as vulval intraepithelial neoplasia (VIN), now also known as high-grade squamous intraepithelial lesion (SIL). The usual type of VIN/SIL is due to HPV. It may be aggravated by smoking.

Vulval lichen sclerosus and chronicerosivelichen planus are skin conditions that also predispose to vulval cancer. They are unrelated to HPV infection.

What are the signs and symptoms?

Signs and symptoms of vulval cancer depend on the type of cancer involved.

Cancer type

Features

Squamous cell carcinoma

Red, pink or white nodule or nodules or plaques

May have a wart-like and/or raw surface if ulcerated

Affected area of vulva may appear white and feel rough

About 50% of women complain of itching or pain

Other symptoms include painful urination, burning, bleeding and discharge not associated with a normal menstrual period

Extramammary Paget disease

Moist red asymmetrical oozing plaque

May cause burning and itching

Basal cell carcinoma

Painless red patch, nodule or ulcer

Melanoma

Appearance of a new pigmented lesion or change in a pre-existing mole

Bartholin's gland cancer

Persistent cystic or thickened tender mass on either side of the opening to the vagina

Diagnosis and staging of vulval cancer

Skin biopsy of the lesion is performed to get an accurate diagnosis of vulval cancer. Under microscopy, the presence of malignant cells along with other histological findings will confirm the diagnosis and the type of vulval cancer.

After initial diagnosis of vulval cancer, a specialist doctor will perform a thorough examination to determine the stage of the cancer. This will determine the size of the tumour, how deeply the tumour has invaded tissues at the site of origin, and the extent of any invasion into surrounding organs or lymph nodes. Determining the cancer's stage is an important factor as it directs what treatment plan should be used. The FIGO (International Federation of Gynaecology and Obstetrics) System of Staging is commonly used to describe vulval cancer staging. The system classifies the disease from Stage 0 through to Stage IV. Stage 0 represents precancerous lesions whilst Stage IV the most advanced stages of cancer (invasion and metastases of surrounding and distant tissues and lymph nodes).

Most patients will have blood tests, a chest X-ray and CT scan of the abdomen and pelvis to help stage the cancer.

What is the treatment of vulval cancer?

Treatment of vulval cancer depends on the type of cancer and the stage of the cancer. In general, diagnosis and treatment during the early stages of cancer have a much better outcome. There are basically 3 types of treatment options available:

Surgery is the most common form of treatment for vulval cancer. Several methods may be used and are often dictated by the stage of the cancer One of these is wide local excision, which takes out the cancer and some of the normal tissue around the cancer. Another is radical local excision which takes out the cancer and about a 1cm portion of surrounding tissue. The lymph nodes in the groin are usually removed when the cancer is 1mm or greater in depth.

Complications of surgery often relate to destruction of the lymphatic channels in the groin and include lymphocysts and lymphoedema.

How can vulval cancer be prevented?

HPV vaccination is expected to reduce the incidence of vulval cancer in the future. Quadrivalent HPV vaccination (Gardisil™) protects against HPV 16, 18, 6 and 11. In New Zealand, funded vaccination has been offered to adolescent girls since 2006.

Most sexually active men and women will acquire at least one subtype of genital HPV, the majority within the first 5 years of onset of sexual activity. For maximum efficacy, vaccination needs to be undertaken before the onset of sexual activity. HPV infection rates can be reduced by use of condoms during intercourse and limiting the number of sexual partners.

HPV infection can also lead to cervical intraepithelial neoplasia (CIN), anal intraepithelial neoplasia (AIN), vaginal intraepeithelial neoplasia (VAIN) or another invasive cancer of the genital tract such as anus.

It is important for all women to have regular cervical smears according to the national screening programme. These offer an opportunity for regular vulval examinations to detect vulval cancer early.

Women with vulval lichen sclerosus and vulval erosive lichen planus should arrange checks with their doctor every year or two, and earlier if they are aware of an enlarging lump or persistent sore.